대한흉부영상의학회 Weekly Chest Cases

Case No : 922 Date 2015-06-29

Courtesy of Ji Eun Lee, Sang Hyun Paik, Jai Soung Park / Soonchunhyang University Bucheon Hospital
Age/Sex 16 / M
Chief ComplaintAbnormal findings on chest radiograph

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Diagnosis With Brief Discussion

Diagnosis: Lymphangiomatosis and Gorham’s disease
Radiologic Findings: • Chest radiograph (Fig. 1) shows multiple expansile osteolytic lesions with sclerotic margin at posterior arc of right 5th, 6th 8th , left 3rd and 4th ribs, which suggests differential diagnosis such as fibrodysplasia or osteolytic bone metastasis. Also, the left paratracheal stripe is widened, possibly due to space occupying lesions such as left paratracheal lymphadenopathy or mediastinal mass. There is no evidence of abnormal parenchymal lesions in both lungs. Chest CT scan (Fig. 2) reveals multiple expansile osteolytic lesions in right 8th , left 3rd and 4th ribs, (Fig. 3) a low density non-enhancing cystic mass in the posterior mediastinum, and (Fig. 4) multiple small low density cystic lesions in the spleen. These findings are compatible with lymphangiomatosis, presenting as cystic lymphangioma of the posterior mediastinum and lymphangiomatosis of spleen, with additional diagnosis of Gorham’s disease due to the bony involvement.
Brief Review: • Lymphangiomatosis is a rare disorder with poorly understood etiology characterized by proliferation of the lymphatic vessels. Various different sites may be involved such as the dermis, soft tissues, bone and viscera.
• Bony involvement of the lymphangiomatosis may give an additional differential diagnosis of Gorham’s disease. These two conditions are understood as a spectrum of the lymphangiomatosis process, rather than a separate disease entity.
• Due to the non-malignant proliferation of vascular or lymphatic vessels within the bone, Gorham’s disease leads to resorption and replacement of the involved bone by fibrovascular connective tissue. Thus, it is also known as idiopathic massive osteolysis, phantom bone disease or disappearing bone disease.
• Gorham’s disease may occur at any age, but is most frequently recognized in children and young adults, with no sex predilection or inheritance pattern. The clinical presentation may be variable, depending on the site of involvement, but usually involves pain, fracture and extremity weakness. Radiological investigation is needed for initial diagnosis, continued management and long-term follow-up.
• Bone lesions in Gorham’s disease occurs in majority of patients and usually involves maxillofacial, upper extremity and trunk. First, it appears as an intramedullary and subcortical radiolucent foci which eventually leads to concentric shrinkage and tapering in tubular bones. More progressed disease shows cortical erosion along with local invasion of angiomatous mass into the adjacent soft tissue, which results in disappearance of remaining bone and progression of osteolytic process to multiple contiguous bones.
• Bone involvement in Gorham’s disease should be differentiated with local or systemic conditions associated with bone and cartilage destruction, such as metastatic tumor, hyper-parathyroidism, gout or pseudarthrosis.
• Thoracic involvement of the Gorham’s disease includes widespread involvement of the skeleton, lymphangioma, chylothorax and rarely, lung parenchymal abnormalities such as diffuse interstitial marking or edema.
• Chylothorax is due to invasion of the thoracic duct or penetration of the pleural cavity by dysplastic lymphatic vessels. It may be recurrent and even fatal when bilateral.
• Mediastinal lymphangioma associated with Gorham’s disease is rare, but may appear as a well-defined, homogenously low attenuated mass with no mass effect.
References: • Jannette C. Case 92: Gorham Syndrome. Radiology 2006; 238:1066–1069
• Yoo SY, Goo JM, Im JG. Mediastinal Lymphangioma and Chylothorax: Thoracic Involvement of Gorham’s Disease. Korean J Radiol 2002;3:130-132
• Kotecha R, Mascarenhas L, Jackson HA, Venkatramani R. Radiological features of Gorham’s disease. Clinical Radiology 67 2012;782-788
• Gordon KD., Mortimer PS. Progressive Lymphangiomatosis and Gorham’s disease: Case report and Clinical implications. Lymphatic research and biology 2011;9:201-204
Keywords: Mediastinum, Multiple organ, Congenital, Vascular malformation,

No. of Applicants : 81

▶ Correct Answer : 48/81, 59.3%

- McGill University Health Center , Canada Alexandre Semionov
- Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
- Niigata University , Japan Atsushi Uehara
- university of montreal , Canada Andrei Gorgos I
- Niigata City General Hospital , Japan Takao Kiguchi
- Government Medical College, Nagpur, India , India Krishna Prasad Bellam
- Santosh hospital,Bengaluru , India Pravin Mahadevappa
- The University of Tokyo Hospital , Japan Takeyuki Watadani
- Kyoto University , Japan Akihiko Sakata
- The University of Tokyo Hospital , Japan Toshihiro Furuta
- Seoul university hospital , Korea (South) Youngjin Ryu
- Juntendo University Hospital , Japan Akifumi Hagiwara
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- Tong young Red cross hospital , Korea (South)
- Bahcesehir University School of Medicine, Radiology Department , Turkey Mustafa Kemal Demir
- Ewha Womans University , Korea (South) Sung Shine Shim
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Shiga University of Medical Science , Japan Akitoshi Inoue
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Okayama University Hospital , Japan Toshiyuki Komaki
- CHU Poitiers , France CHAN paul
- Daejin Medical Center Bundang Jesaeng General Hospital , Korea (South) Donghwan Kim
- Chonnam National University Hospital , Korea (South) So yeon Ki
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Asan Medical Center , Korea (South) Hyung Jung Koo
- Asan Medical Center , Korea (South) Soyeoun Lim
- Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
- 異⑸ , Korea (South) Yunhee Jang
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Chonnam national university hospital , Korea (South) INSOO JANG
- CNUH , Korea (South) LEE EUN
- Asan , Korea (South) Sania ALBlushi
- Fortis hospital , Mohali , India Shaleen Rana
- Freelance resident in Reunion Island , Reunion Fabien HO
- Goa Medical College , India Paresh Desai
- ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
- National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida
- Okinawa Kyodo Hospital , Japan Shoko Iraha
- Gifu Central Hospital , Japan Haruo Watanabe
- IRSA La Rochelle , France Denis Chabassiere
- NASA SCANS NEW DELHI , India RAKESH BHATIA
- Northern Yokohama Showa university , Japan Kota Watanabe
- the first affiliatited hospital of nanjing medical univercity , China Hai Xu
- Otsu Red Cross Hospital , Japan Hirotsugu Nakai
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- chp st martin , France Mariotte benoit
- Pusan National University Hospital , Korea (South) LEE YOU JIN
- Asan Medical Center , Korea (South) , Korea (South) Han Na Lee

▶ Correct Answer as Differential Diagnosis : 2/81, 2.5%

- Osaka Rosai Hospital , Japan Hiromitsu Sumikawa
- CLCC Rennes , France nicolas gautier

▶ Semi-Correct Answer : 2/81, 2.5%

- Armed Force Seoul Hospital , Korea (South) Eui Jin Hwang
- Beaulieu clinic Geneva , Switzerland gilles GENIN

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 922 Date 2015-06-29

Discussion

No. of Applicants : 81